Gastrointestinal manifestations of Systemic Lupus Erythematosus: What needs to be considered in an acute setting?

Clinical History

A patient with a known case of SLE, chronic kidney disease, presented to the ER with worsening abdominal pain of 1-day duration with a bloody tinge in stool. Laboratory tests showed anaemia (Hb-8gm/dl), raised ESR-23 mm/h, Prolonged PTT, Anticardiolipin and ANA positive, raised creatinine-1.8 and normal liver function tests. No history of prior anticoagulants.

Imaging Findings

Computed Tomography (CT) of the abdomen with contrast (Fig-1) revealed long segment concentric wall thickening of the ascending colon until the mid transverse colon, giving a target appearance in axial sections. The inner mural layers appeared hyperdense on plain scan, consistent with intramural haemorrhage. The superior mesenteric vessels were patent. There was also hyperdensity (70HU) tracking along the rectus sheath, suggestive of rectus sheath hematoma. The CT features of rectus sheath hematoma and long segment concentric wall thickening was attributed to lupus vasculits.

Discussion

Background:

Systemic lupus erythematosus (SLE) is a chronic immune mediated multisystemic disorder commonly affecting women aged between 20 and 45 years, but may also affect both sexes and all age groups including children. The aetiology is largely unknown, however the role of viral, hormonal, environmental and genetic factors are attributed. American college of Rheumatology (ACR) classification criteria suggests at least 4 or more of the 11 features (Malar rash, Discoid rash, Photosensitivity, Oral ulcers, Arthritis, Serositis, Renal disorder, Neurologic disorder, Hematologic disorder, Immunologic disorder, Antinuclear antibody-ANA) are needed for a diagnosis of SLE.

Clinical Perspective:

The reported incidence of gastrointestinal manifestations of SLE varies between 1.3% and 27.5% [1]. The most common symptomatic presentation of abdominal manifestation of SLE is anorexia, nausea and vomiting. However patients may also present with dysphagia, abdominal pain, malena, diarrhoea or chronic constipation [2]. Although only renal involvement forms part of diagnostic criteria amongst solid organs in the abdomen, effort should be made to rule out multi-organ abdominal involvement when the patient presents with abdominal symptoms [3]. The approach to an SLE patient presented with acute abdomen is summarised in fig-3.

Imaging perspective: (Fig-2)

Abdominal imaging manifestations [2-6] are summarised in fig:2. However, radiological differentiation of abdominal SLE related vasculitic manifestations from other causes is challenging. In lupus, the medium or small vessel disease will combine with atherosclerotic, thrombotic disease resulting in auto-inflammatory driven degeneration of the wall or may co-exist with SLE related drug/infection/autoimmune process [7]. Thus biopsy would be necessary to ascertain the cause of pathology, and a history of SLE must never be overlooked.

Outcome:

Patients might need prompt high dose steroids and immunosupressants, once the possibility of infection and other primary diseases are ruled out. If the patient fails to respond to medical management and or deteriorates, surgical resection of the non-viable bowel segments would improve the prognosis.

In our case, a diagnostic laparoscopy was done, showing normal appearing bowel loops with non-expanding rectus sheath hematoma with biopsy confirming lupus vasculitis. The patient was started on high dose steroids and immunosuppressive agents. The patient improved symptomatically and was discharged a week later.

Teaching points:

1. Gastrointestinal manifestations of SLE are not uncommon, and may mimic other primary abdominal organ involvement if history of an underlying SLE is overlooked.
2. SLE patients presenting with a gastrointestinal symptom, drug related side effects, infective causes or vasculitic sequalae may occur.

Differential Diagnosis List

Final Diagnosis

Lupus vasculitis with ischemia of ascending colon.Rectus sheath hematoma.

URL:	https://eurorad.org/case/14426
DOI:	10.1594/EURORAD/CASE.14426
ISSN:	1563-4086